Background: Fetal adenocarcinoma of the lung is a rare subtype of pulmonary adenocarcinoma with a relative estimated incidence of 0.5% or fewer of all lung cancers. Because of its extreme rarity, there have been no controlled clinical trials investigating treatment regimens for fetal adenocarcinoma and, as a result, there are no guidelines for management. Case presentation: We report a case of a well-differentiated fetal adenocarcinoma, which is a variant of pulmonary blastoma, that is a low-grade malignancy and associated with a good prognosis. A 29-year-old Moroccan man presented with a well-differentiated fetal adenocarcinoma staged T3N0M0, who received 3 cycles of neoadjuvant chemotherapy followed by surgery, with no recurrence at 2 years follow-up. Conclusion: Fetal adenocarcinoma is a rare suptype of adenocarcinoma. Surgical resection is the treatment of choice for resectable disease. The role of chemotherapy in the neoadjuvant setting or adjuvant setting is not well defined.